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Kidney cancer is a frequent malignant disease. To date, there is no evidence on the effectiveness of early detection and, in most cases, surgery represents the only standard treatment. So far, there is no standardized therapy for localized and locally advanced renal tumors; however, the recent introduction of target therapy has significantly improved the prognosis of metastatic disease. Therefore, survival differences in Europe are deemed to involve differences in diagnostic and therapeutic approaches. The aim of the SUDCAN collaborative study was to compare the net survival from kidney cancer between six European Latin countries (Belgium, France, Italy, Portugal, Spain, and Switzerland) and provide trends in net survival and dynamics of excess mortality rates up to 5 years after diagnosis. The data were extracted from the EUROCARE-5 database. First, net survival was studied over the 2000-2004 period using the Pohar-Perme estimator. For trend analysis, the study period was specific to each country. The results are reported from 1992 to 2004 in France, Italy, Spain, and Switzerland, and from 2000 to 2004 in Belgium and Portugal. These analyses were carried out using a flexible excess rate modeling strategy. In 2000-2004, the 5-year net survival ranged between 59% (Spain) and 67% (France and Italy) in men and between 60% (Spain) and 73% (Portugal) in women. There was an increase in the age-standardized net survival between 1992 and 2004 at 1 year, as well as at 5 years, in all age groups and countries. Irrespective of the year of diagnosis, the excess mortality rate decreased with time elapsed since diagnosis. There are some differences in survival from kidney cancer between European Latin countries, but a considerable improvement was observed in most countries.

Liver cancer represents a major clinical challenge. The aim of the SUDCAN collaborative study was to compare the net survival from liver cancer between six European Latin countries (Belgium, France, Italy, Portugal, Spain and Switzerland) and provide trends in net survival and dynamics of excess mortality rates (EMRs) up to 5 years after diagnosis. The data were extracted from the EUROCARE-5 database. First, net survival was studied over the period 2000-2004 using the Pohar-Perme estimator. For trend analyses, the study period was specific to each country. Results are reported from 1992 to 2004 in France, Italy, Spain and Switzerland and from 2000 to 2004 in Belgium and Portugal. These trend analyses were carried out using a flexible excess-rate modeling strategy. There were little differences between the six countries in the 5-year age-standardized net survival (2000-2004): it ranged from 13% (France and Portugal) to 16% (Belgium). An increase in the net age-standardized survival was observed in all countries between 1992 and 2004, both at 1 year and at 5 years (the highest in Spain, the lowest in France). Generally, patients aged 60 years showed the highest increase. There was a progressive decrease in EMR over the 5-year- period following diagnosis. The study confirmed the poor prognosis of liver cancer. Innovative treatments might improve the prognosis as well as preventive screening of cirrhotic patients with good liver function. Efforts are also needed to improve registration practices.

IF 2.415; International audience; Gastric cancers are a clinical challenge. The aim of the SUDCAN collaborative study was to compare the net survival from gastric cancer between six European Latin countries (Belgium, France, Italy, Portugal, Spain and Switzerland) and explore the trends in net survival and in the dynamics of the excess mortality rates (EMRs) up to 5 years after diagnosis. The data were extracted from the EUROCARE-5 database. First, net survival was studied over the period 2000-2004 using the Pohar-Perme estimator. For trend analyses, the study period was specific to each country. The results are reported from 1992 to 2004 in France, Italy, Spain and Switzerland and from 2000 to 2004 in Belgium and Portugal. These trend analyses were carried out using a flexible excess rate modelling strategy. There were little differences between countries in age-standardized net survival for stomach cancer (2000-2004). The 5-year net survival ranged between 26 (Spain) and 32% (Italy). There was a small increase in the age-standardized net survival at 1 year between 1992 and 2004. The increase was also observed in the 5-year net survival, except in France, where the increase was less marked. A slight decrease in the EMR between 1992 and 2004 was limited to the 24 months after diagnosis. In addition, the decrease in the EMR was the same whatever the year of diagnosis. There were minor differences in survival from stomach cancer between European Latin countries. A slight improvement in the 5-year net survival was observed in all countries and the major gain was observed during the 24 months after diagnosis. Development of innovative treatments is needed to improve the prognosis. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

IF 2.415; International audience; Colon cancer represents a major public health issue. The aim of the SUDCAN collaborative study was to compare the net survival from colon cancer between six European Latin countries (Belgium, France, Italy, Portugal, Spain, and Switzerland) and provide trends in net survival and dynamics of the excess mortality rates up to 5 years after diagnosis. The data were extracted from the EUROCARE-5 database. First, net survival was studied over the 2000-2004 period using the Pohar-Perme estimator. For trend analyses, the study period was specific to each country. Results were reported from 1992 to 2004 in France, Italy, Spain, and Switzerland and from 2000 to 2004 in Belgium and Portugal. These analyses were carried out using a flexible excess rate modeling strategy. There were few differences between countries in age-standardized net survivals (2000-2004). During the 2000-2004 period, the 5-year net survival ranged between 57 (Spain and Portugal) and 61% (Belgium and Switzerland). The age-standardized survival at 1 and 5 years after diagnosis increased between 1992 and 2004. This increase was observed at ages 60 and 70, but was less marked at 80. This increase was linked to a marked decrease in the excess mortality rate between 1992 and 2004 until 18 months after diagnosis. Beyond this period, the decrease in the excess mortality rates among countries was modest and nearly the same whatever the year of diagnosis. There were minor differences in survival after colon cancer between European Latin countries. A considerable improvement in the 5-year net survival was observed in all countries, but the gain was mainly limited to the first 18 months after diagnosis. Further improvements are expected through the implementation of mass screening programs.

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The main objective of the SUDCAN study was to compare, for 15 cancer sites, the trends in net survival and excess mortality rates from cancer 5 years after diagnosis between six European Latin countries (Belgium, France, Italy, Portugal, Spain and Switzerland). The data were extracted from the EUROCARE-5 database. The study period ranged from 6 (Portugal, 2000-2005) to 18 years (Switzerland, 1989-2007). Trend analyses were carried out separately for each country and cancer site; the number of cases ranged from 1500 to 104 000 cases. We developed an original flexible excess rate modelling strategy that accounts for the continuous effects of age, year of diagnosis, time since diagnosis and their interactions. Nineteen models were constructed; they differed in the modelling of the effect of the year of diagnosis in terms of linearity, proportionality and interaction with age. The final model was chosen according to the Akaike Information Criterion. The fit was assessed graphically by comparing model estimates versus nonparametric (Pohar-Perme) net survival estimates. Out of the 90 analyses carried out, the effect of the year of diagnosis on the excess mortality rate depended on age in 61 and was nonproportional in 64; it was nonlinear in 27 out of the 75 analyses where this effect was considered. The model fit was overall satisfactory. We analysed successfully 15 cancer sites in six countries. The refined methodology proved necessary for detailed trend analyses. It is hoped that three-dimensional parametric modelling will be used more widely in net survival trend studies as it has major advantages over stratified analyses.

Pancreatic cancer represents a real clinical challenge. The aim of the SUDCAN collaborative study was to compare the net survival from pancreatic cancer between six European Latin countries (Belgium, France, Italy, Portugal, Spain and Switzerland) and provide trends in net survival and dynamics of excess mortality rates up to 5 years after diagnosis. The data were extracted from the EUROCARE-5 database. First, net survival was studied over the period 2000-2004 using the Pohar-Perme estimator. For trend analyses, the study period was specific to each country. Results were reported from 1992 to 2004 in France, Italy, Spain and Switzerland and from 2000 to 2004 in Belgium and Portugal. These analyses were carried out using a flexible excess rate modelling strategy. There were little differences between countries in age-standardized net survivals (2000-2004). The 5-year net survival was poor (range: 6-10%). The changes in net survival from 1992 to 2004 were mostly related to early survival and patients aged 60 years. A slight decrease in the excess mortality rate between 1992 and 2004 was observed, limited to the 18 months after diagnosis. This study confirmed that, despite some improvement, survival from pancreatic cancer is still poor throughout European Latin countries. The major improvements in clinical imaging did not result in improvements in prognosis. Development of truly innovative treatments is highly needed to improve prognosis. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

Rectal cancer is a common and serious disease. The aim of the SUDCAN collaborative study was to compare the net survival from rectal cancer between six European Latin countries (Belgium, France, Italy, Portugal, Spain, and Switzerland) and provide trends in net survival and dynamics of excess mortality rates up to 5 years after diagnosis. The data were extracted from the EUROCARE-5 database. First, net survival was studied over the period 2000-2004 using the Pohar-Perme estimator. For trend analyses, the study-period was specific to each country. The results are reported from 1992 to 2004 in France, Italy, Spain, and Switzerland and from 2000 to 2004 in Belgium and Portugal. These analyses were carried out using a flexible excess rate modeling strategy. There were some differences between countries in age-standardized net survivals (2000-2004). The 5-year survival ranged from 55% (Portugal) to 62% (Belgium). There was an increase in age-standardized survival rates between 1992 and 2004 as observed at 1 and 5 years. This increase was observed in the 60 and 70-year age groups, but was less marked in the 80-year age group. This was related to a decrease in the excess mortality rates between 1992 and 2004, until ∼24 months after diagnosis in France and Switzerland, whereas it was continuous over the entire study period in Italy and Spain. Considerable improvements in survival from rectal cancer have been achieved. Further improvements are expected through better adherence to the guidelines and the implementation of mass screening.

Corpus uteri cancer is the most common gynaecological cancer in women in Europe, but presents a relatively good prognosis. There were two main objectives in this study: estimate differences between countries in age-standardized net survival (NS) at 1 and 5 years in 2000-2004 and evaluate time trends in NS and excess mortality rates in 1992-2004. Data on corpus uteri malignant tumours (International Classification of Diseases for Oncology, third ed.: C54) were extracted from the EUROCARE database for six European Latin countries: Belgium, France, Italy, Portugal, Spain and Switzerland. NS was estimated for each country using the nonparametric estimator proposed by Pohar-Perme. Trends in NS and excess mortality rates up to 5 years after diagnosis were assessed using a multivariable parametric flexible modelling. The study analysed 25 508 cases for the first objective and 43 550 for the second. Age-standardized 1-year NS ranged from 88% (Portugal and Spain) to 93% (Switzerland), whereas 5-year survival ranged between 72% (Portugal) and 79% (Belgium and Switzerland). From 1992 to 2004, the NS increased in all countries with available information on this period (France, Italy, Spain and Switzerland). Also, in Belgium and Portugal, there was an increase in NS between 2000 and 2004. Improvements in survival were more evident for older ages (75 years). There were some differences in NS between the countries studied (maximum of 5% at 1 year and 7% at 5 years). The NS improved in all countries during the period studied and the differences between countries narrowed.

In Europe as a whole, survival from skin malignant melanoma (SMM) has increased constantly since the 1980s. The aim of the SUDCAN collaborative study was to compare the trends in the 5-year net survival from SMM and in related excess mortality rate between six European Latin countries (Belgium, France, Italy, Portugal, Spain, and Switzerland). The data were extracted from the EUROCARE-5 database (end of follow-up: 01 January 2009). First, the net survival was studied over the 2000-2004 period using the Pohar-Perme estimator. For trend analyses, the study period was specific to each country. The results are reported from 1992 to 2004 in France, Italy, Spain, and Switzerland and from 2000 to 2004 in Belgium and Portugal. The analyses were carried out using a flexible excess rate modeling. Over the 2000-2004 period, the 5-year net survival from SMM ranged from 79 (Portugal) to 90% (Switzerland). In all countries, net survival was higher in women versus men and in young versus old age groups. From 1992 to 2004, the 5-year net survival increased the most in the countries with the lowest survivals in 1992 (+9% in Italy and Spain vs. +2% in Switzerland or +4% in France). The differences between countries decreased between 1992 and 2004. Although survival increased to a lower or higher extent in all countries during the period studied, significant differences in net survival from SMM persisted among the six countries studied. Health policies should mainly enhance early diagnosis by increasing public awareness and with screening campaigns. Furthermore, new immunotherapies, which will be approved soon hopefully, should also be used to improve the outcomes of SMM treatment.

The aim of the SUDCAN collaborative study was to compare the trends in 1- and 5-year net survival and the trends in the dynamics of the excess mortality rates in head and neck cancers between six European Latin countries (Belgium, France, Italy, Portugal, Spain and Switzerland). The data were extracted from the EUROCARE-5 database. First, the net survival was studied over the 2000-2004 period using the Pohar-Perme estimator. For trend analyses, the study period was specific to each country. The results are reported from 1992 to 2004 in France, Italy, Spain and Switzerland and from 2000 to 2004 in Belgium and Portugal. The analyses were carried out using a flexible excess rate modelling. There were significant differences between countries in 5-year age-standardized net survivals over the 2000-2004 period, ranging from 33 to 34% in France and Portugal from 42 to 44% in Switzerland and Italy, respectively. The age-standardized net survival improved considerably from 1992 to 2004 in Italy, Spain and Switzerland, but not in France because of lack of improvement in the elderly. The increase in net survival was linked to a decrease in the excess mortality rate up to 3-4 years after diagnosis. The net survival from head and neck cancers improved over the study period, but significant differences were still observed in 2004. Differences in sex ratio and anatomical distribution contributed only partially towards these disparities. Differences in stage at diagnosis, time to treatment and/or proportion of human papillomavirus-related cases are also probably involved in the survival disparities observed. Overall, the prognosis of these tumours remains poor.

Cancer survival is a key measure of the effectiveness of a healthcare system. As differences in healthcare systems are present among European Latin countries, it is of interest to look specifically at their similarities and differences in terms of cancer survival. Incident cases were extracted from the EUROCARE-V database for France, Italy, Spain, Switzerland, Portugal, and Belgium. One and 5-year net survivals (NS) were calculated for the period 2000-2004 using the Pohar-Perme estimator. Trends in NS over the 1992-2004 period and changes in the pattern of cancer excess mortality rate until 5 years after diagnosis were examined using a multivariate excess mortality rate model. There were moderate differences in age-standardized NS between countries (5-year NS range: 83-88%), but significant differences in the age groups 15-54 and 55-74 years (at 5 years up to +16 and +18% between any two countries). During the study period, excess mortality and NS improved in Italy, Spain, and Portugal. In Italy and Portugal, this improvement was slightly similar at ages 40, 55, and 70 whereas, in Spain, there was a sharp increase in NS at age 55. Because of this improvement, excess mortality and NS were similar in all six countries in 2004. Excess mortality peaked around 1 year after diagnosis in the youngest ages, but decreased gradually in the elderly. Detailed analyses showed differences in excess mortality and NS from cervical cancer between European Latin countries. However, these differences decreased over the study period because of the considerable improvement in Spain, Italy, and Portugal.

Proportion cured is a potentially more informative cancer outcome measurement than 5-year survival. We present population-based estimates of cure for young patients diagnosed with acute lymphoblastic leukemia in Europe from 1982 to 2002. Thirty-five European cancer registries provided data. Survival was estimated by age, period of diagnosis and European region, and used as input for parametric cure models, which assume cured patients have the same mortality as the general population. For acute lymphoblastic leukemia diagnosed in 1–14 year olds in 2000–2002, over 77% were estimated cured. The proportion cured improved significantly over the study period: an impressive 26–58% in infants (up to 1 year), 70–90% in 1–4 year olds, 63–86% in 5–9 year olds, 52–77% in 10–14 year olds, and 44–50% in 15–24 year olds. Regional variations in proportion cured reduced over time for 1–14 year-olds, but persisted in infants and 15–24 year olds. Five-year survival was always slightly higher than proportion cured. Considerable proportions of young patients were estimated cured of acute lymphoblastic leukemia. Nevertheless, a small excess risk of death persisted beyond five years after diagnosis when patients remained at risk for late treatment effects, late relapses and second primaries.

Embryonal cancers are a heterogeneous group of rare cancers which mainly occur in children and adolescents. The aim of the present study was to estimate the burden (incidence, prevalence, survival and proportion of cured) for the principal embryonal cancers in Europe (EU27), using population-based data from cancer registries (CRs) participating in RARECARE. We identified 3322 cases diagnosed from 1995 to 2002 (latest period for which data are available): 44% neuroblastoma, 35% nephroblastoma, 13% retinoblastoma and 6% hepatoblastoma. Very few cases of pulmonary blastoma (43 cases) and pancreatoblastoma (seven cases) were diagnosed. About 2000 new embryonal cancers were estimated every year in EU27, for an annual incidence rate of 4 per million (1.8 neuroblastoma, 1.4 nephroblastoma, and 0.5 retinoblastoma); 91% of cases occurred in patients under 15 years. Five-year relative survival for all embryonal cancers was 80% (99% retinoblastoma, 90% nephroblastoma, 71% hepatoblastoma and 68% neuroblastoma). Overall survival was lower in adolescents and adults than in those under 15 years. The cure rate was estimated at 80%. Slightly less than 40,000 persons were estimated alive in EU27 with a diagnosis of embryonal cancer in 2008. Nephroblastoma was the most prevalent (18,150 cases in EU27), followed by neuroblastoma (12,100), retinoblastoma (5200), hepatoblastoma (2700) and pulmonary blastoma (614). This is the first study to delineate the embryonal cancer burden in Europe by age, sex and European region. Survival/cure rate is generally high, but there are considerable gaps in our understanding of the natural histories of these rare diseases particularly in adults.

We provide updated estimates of survival, incidence, complete prevalence, and proportion cured for patients with testicular/paratesticular and extragonadal germ cell cancers in Europe, grouped according to the new list of cancer types developed by RARECARE. We collected data, archived in European cancer registries, with vital status information available to 31st December 2003.We analysed 26,000 cases of testicular, paratesticular and extragonadal germ cell cancers diagnosed 1995-2002, estimating that about 15,600 new testicular/paratesticular and 630 new extragonadal cancer cases occurred per year in EU27, with annual incidence rates of 31.5/1,000,000 and 1.27/1,000,000, respectively. Slightly more than 436,000 persons were alive at the beginning of 2008 with a diagnosis of testicular/paratesticular cancer, and about 17,000 with a diagnosis of extragonadal germ cell cancer.Five-year relative survival was 96% for testicular/paratesticular cancer and 71% for extragonadal germ cell cancer; the proportions cured were 95% and 69%, respectively. We found limited variation in survival between European regions except for non-semino matous testicular cancer, for which five-year relative survival ranged from 86% in Eastern Europe to 96% in Northern Europe. Survival for all cancer types considered decreased with increasing age at diagnosis.Further investigation is required to establish the real reasons for the lower survival in Eastern Europe. Considering the high prevalence of these highly curable cancers, it is important to monitor patients long-term, so as to quantify treatment-related risks and develop treatments having limited impact on quality of life. (C) 2011 Elsevier Ltd. All rights reserved.

To access publisher's full text version of this article. Please click on the hyperlink in Additional Links field. Survival and cure rates for childhood cancers in Europe have greatly improved over the past 40 years and are mostly good, although not in all European countries. The EUROCARE-5 survival study estimates survival of children diagnosed with cancer between 2000 and 2007, assesses whether survival differences among European countries have changed, and investigates changes from 1999 to 2007. We analysed survival data for 157,499 children (age 0-14 years) diagnosed between Jan 1, 1978 and Dec 31, 2007. They came from 74 population-based cancer registries in 29 countries. We calculated observed, country-weighted 1-year, 3-year, and 5-year survival for major cancers and all cancers combined. For comparison between countries, we used the corrected group prognosis method to provide survival probabilities adjusted for multiple confounders (sex, age, period of diagnosis, and, for all cancers combined without CNS cancers, casemix). Age-adjusted survival differences by area and calendar period were calculated with period analysis and were given for all cancers combined and the major cancers. We analysed 59,579 cases. For all cancers combined for children diagnosed in 2000-07, 1-year survival was 90.6% (95% CI 90.2-90.9), 3-year survival was 81.0 % (95% CI 80.5-81.4), and 5-year survival was 77.9% (95% CI 77.4-78.3). For all cancers combined, 5-year survival rose from 76.1% (74.4-77.7) for 1999-2001, to 79.1% (77.3-80.7) for 2005-07 (hazard ratio 0.973, 95% CI 0.965-0.982, p

This paper describes the usage and the performance evaluation of the completeness index method in the ‘Surveillance of Rare Cancers in Europe project’ (RARECARE) for estimating rare cancer prevalence in Europe. The 15-year prevalence at 1st January 2003 for 255 cancers is obtained from a pool of 22 RARECARE cancer registries (CRs). Incidence and survival models are applied to the RARECARE database to estimate the parameters from which the completeness indices are calculated. Complete prevalence is obtained adjusting the observed 15-year prevalence by the completeness index, to account for those cancer survivors diagnosed before the CR activity started. Main factors influencing the performance of the completeness index method for rare cancers are the same as for common cancers: age distribution of incidence and lethality of the cancer. For cancers occurring in the elderly, with low survival rates and consequently a restricted number of long-term survivors we obtained completeness indices higher than 0.9. Values lower than 0.7 correspond to those cancers with good prognosis and/or incidence more concentrated at the younger ages, indicating that 15 years of follow up are insufficient to detect all prevalent cases. Validation analysis shows that for a restricted subgroup of rare cancers with very low incidence and low survival, the completeness indices were not able to adequately correct the observed prevalence even considering a registration period of 20 years. On average, sensitivity analyses show a slight overestimation of complete prevalence for rare and common cancers whose increasing incidence is known in literature. RARECARE is the largest project on rare cancers conducted to date. Improving health care programs for cancer survivors is a public health priority and prevalence data which provides important information in this field should be regularly asked to Member States and included in the EU health statistics.

Relevant information on cancer is available, but its organisation and harmonisation is necessary to make it usable for health planners, doctors, patients and other stakeholders. - Cancer registries should be at the core of a European Cancer Information System (ECIS), providing basic cancer indicators on incidence, survival, prevalence and patterns of care but these data need to be systematically linked to clinical, socioeconomic and population data. - The first step towards an ECIS, the harmonisation of incidence and survival data, has been taken during EPAAC in order to update European cancer data and construct a common database computing incidence, survival and prevalence data. - Specific conditions for data use still need to be considered, regulating confidentiality and ownership.

Because of the low incidence, and limited opportunities for large patient volume experiences, there are very few relevant studies of neuroendocrine tumours (NETs).A large population-based database (including cancer patients diagnosed from 1978 to 2002 and registered in 76 population-based cancer registries [CRs]), provided by the project 'surveillance of rare cancers in Europe' (RARECARE) is used to describe the basic indicators of incidence, prevalence and survival of NETs, giving a unique overview on the burden of NETs in Europe. NETs at all cancer sites, excluding lung, were analysed in this study. In total over 20,000 incident cases of NETs were analysed and a data quality check upon specific NETs was performed. The overall incidence rate for NETs was 25/1,000,000 and was highest in patients aged 65 years and older with well differentiated endocrine carcinomas (non-functioning pancreatic and gastrointestinal) (40 per 1,000,000). We estimated that slightly more than 100,000 people were diagnosed with NETs and still alive in EU27 at the beginning of 2008. Overall, NETs had a 5 year relative survival of 50%; survival was low (12%) for poorly differentiated endocrine carcinoma, and relatively high (64%) for well differentiated carcinoma (not functioning of the pancreas and digestive organs). Within NETs, endocrine carcinoma of thyroid gland had the best 5-year relative survival (82%).Because of the complexity and number of the different disciplines involved with NETs (as they arise in many organs), a multidisciplinary approach delivered in highly qualified reference centres and an international network between those centres is recommended. (c) 2013 Elsevier Ltd. All rights reserved.

International audience; BACKGROUND AND AIMS: Little is known about the epidemiology of rare epithelial digestive cancers. The aim of this study was to report on their incidence, prevalence and survival across Europe. METHODS: The analysis was carried out on 50,646 cases diagnosed from 1995 to 2002 within a population of 162,000,000 in 21 European countries. Age-standardised incidence rates were computed using the European standard population. Prevalence rates, relative survival and period survival indicators for the years 2000-2002 were calculated. The expected number of new cases per year and of prevalent cases in Europe was estimated. RESULTS: There were large variations in gallbladder epithelial cancer incidence rates: the incidence in Eastern Europe was 7 times higher than in the UK & Ireland. Differences between incidence rates were smaller for the other sites. The estimated number of new epithelial cancers arising in the EU each year was estimated to be 11,050 for extrahepatic bile duct cancer, 10,713 for gallbladder cancer, 5427 for anal cancer and 3595 for small intestine cancer. The corresponding estimated number of total prevalent cases was 18,483, 15,620, 40,589 and 13,276. There was also a large variation in the 5-year relative survival rate. For epithelial cancer of the anal canal, this varied between 66% (Central Europe) and 44% (Eastern Europe). The corresponding rates for small intestine cancers were 33% and 20%, for extrahepatic bile duct cancers, 17% and 12% and for gallbladder cancer 13% and 10%. CONCLUSION: There are large variations within Europe in the incidence and survival of rare digestive cancers according to geographic area.

Background The Surveillance of Rare Cancers in Europe (RARECARE) project aims at increasing knowledge of rare cancers in Europe. This manuscript describes the epidemiology of myeloid malignancies (MMs), taking into account the morphological characterisation of these tumours. Methods We used data gathered by RARECARE on cancer patients diagnosed from 1995 to 2002 and archived in 64 European population-based cancer registries, followed up to 31st December 2003 or later. Results The overall annual crude incidence of MMs was 8.6 per 100,000. Acute myeloid leukaemia (AML) and myeloproliferative neoplasms (MPN) were most common, with incidence rates of 3.7 and 3.1 per 100,000 year respectively, followed by 1.8 for myelodysplastic syndromes (MDS) and myelodysplastic/myeloproliferative neoplasms (MD/MPN) and 0.1 for histiocytic and dendritic cell neoplasms (HDCN). The 5-year relative survival rate ranged from 18% for chronic myelomonocytic leukaemia, 19% for AML, 29% for MDS and 44% for chronic myeloid leukaemia to relatively favourable rates for MPN (62%) and HDCN (83%). Total number of new cases of MMs in the EU27 is estimated at 43,000 annually, total number of prevalent cases (1st January 2008) at 189,000 cases. Conclusion MMs form a large variety of rare entities with specific characteristics. Collection of detailed information (immunophenotype, genetic abnormalities, molecular data and clinical data) and an up-to-date classification system is essential for their surveillance, especially now that more and more targeted therapies are being introduced.

The rarity or the asymptomatic character of endocrine tumours results in a lack of epidemiological studies on their incidence and survival patterns. The aim of this study was to describe the incidence, prevalence and survival of endocrine tumours using a large database, which includes cancer patients diagnosed from 1978 to 2002, registered in 89 population-based cancer registries (CRs) with follow-up until 31st December 2003. These data give an unique overview of the burden of endocrine carcinomas in Europe.A list of tumour entities based on the third International Classification of Diseases for Oncology was provided by the project Surveillance of rare cancer in Europe (RARECARE) project. Over 33,594 cases of endocrine carcinomas were analysed in this study.Incidence rates increased with age and were highest in patients 65 years of age or older. In 2003, more than 315,000 persons in the EU (27 countries) were alive with a past diagnosis of a carcinoma of endocrine organs. The incidence of pituitary carcinoma equalled four per 1,000,000 person years and showed the strongest decline in survival with increasing age. Thyroid cancer showed the highest crude incidence rates (four per 100,000 person years) and was the only entity with a gender difference: (female-to-male ratio: 2:9). Parathyroid carcinoma was the rarest endocrine entity with two new cases per 10,000,000 person years. For adrenal carcinoma, the most remarkable observations were a higher survival for women compared to men (40% compared to 32%, respectively) and a particularly low relative survival of 24% in patients 65 years of age or older. More high quality studies on rare cancers, with additional information, e. g. on stage and therapeutic approach, are needed and may be of help in partly explaining the observed variation in survival. (c) 2012 Elsevier Ltd. All rights reserved.

To the central nervous system (CNS) belong a heterogeneous group of glial and non glial rare cancers. The aim of the present study was to estimate the burden (incidence, prevalence, survival and proportion of cured) for the principal CNS cancers in Europe (EU27) and in European regions using population-based data from cancer registries participating in the RARECARE project. We analysed 44,947 rare CNS cancers diagnosed from 1995 to 2002 (with follow up at 31st December 2003): 86.0% astrocytic (24% low grade, 63% high grade and 13% glioma NOS), 6.4% oligodendroglial (74% low grade), 3.6% ependymal (85% low grade), 4.1% Embryonal tumours and 0.1% choroid plexus carcinoma. Incidence rates vary widely across European regions especially for astrocytic tumours ranging from 3/100,000 in Eastern Europe to 5/100,000 in United Kingdom and Ireland. Overall, about 27,700 new rare CNS cancers were estimated every year in EU27, for an annual incidence rate of 4.8 per 100,000 for astrocytic, 0.4 for oligodendroglial, 0.2 for ependymal and embryonal tumours and less than 0.1 for choroid plexus carcinoma. More than 154,000 persons with rare CNS were estimated alive (prevalent cases) in the EU at the beginning of 2008. Five-year relative survival was 14.5% for astrocytic tumours (42.6% for low grade, 4.9% for high grade and 17.5% for glioma NOS), 54.5% for oligodendroglial (64.9% high grade and 29.6% low grade), 74.2% for ependymal (80.4% low grade and 36.6% high grade), 62.8% for choroid plexus carcinomas and 56.8% for embryonal tumours. Survival rates for astrocytic tumours were relatively higher in Northern and Central Europe than in Eastern Europe and in UK and Ireland. The different availability of diagnostic imaging techniques and/or radiation therapy equipment across Europe may contribute to explain the reported survival differences. The estimated proportion of cured patients was 7.9% for the 'glial' group to which belong astrocytic tumours. Overall results are strongly influenced by astrocytic tumours that are the most common type. This is the first study to delineate the rare CNS cancer burden in Europe by age, sex and European region.

The RARECARE project has proposed a different and more detailed grouping of cancers, based on localisation and histological type, in order to identify rare entities with clinical meaning. RARECARE gathered data on cancer patients diagnosed from 1978 to 2002 and archived in 76 population-based cancer registries, all of which had vital status information available up to at least 31st December 2003. This study provides incidence, prevalence and survival rates for rare head and neck epithelial (H&N) cancers.Among the rare H&N cancers, those of oral cavity had the highest annual crude incidence rate of 48 per million, followed by oropharynx and 'major salivary glands and salivary gland type tumours' (28 and 13 per million, respectively). Incidence rates of epithelial tumours of nasal cavities, nasopharynx, eye and adnexa and middle ears were all lower than 5 per million. The prevalence for all investigated entities was lower than 35 per 100,000. The 5-year relative survival rates ranged from 40% for epithelial cancer of oropharynx to 85% for epithelial cancer of eye and adnexa. Survival rates were lower for men and for patients aged >= 65 years. With few exceptions, the lowest and highest survival figures were observed for Eastern Europe and Northern Europe, respectively.According to the definition for rare tumours by RARECARE (incidence